Kanitthamniyom Chanakarn, Osorio Alejandra, Saowapa Sakditad, Siladech Pharit
Hematology, Thainakarin Hospital, Bangkok, THA.
Internal Medicine, University of Alabama at Birmingham (UAB) Hospital, Birmingham, USA.
Cureus. 2023 Nov 28;15(11):e49564. doi: 10.7759/cureus.49564. eCollection 2023 Nov.
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of non-Hodgkin lymphoma that manifests as panniculitis-like skin lesions. It frequently co-occurs with hemophagocytic lymphohistocytosis, a life-threatening hyperinflammatory syndrome. The majority of SPTL cases express αβ T-cell receptors (SPTL-AB) and have a favorable prognosis with oral immunosuppressive agents. We report a 37-year-old male patient with HIV infection who had a history of low-grade fever for one year, multiple tender subcutaneous nodules on both thighs, and cytopenia. He received several courses of antibiotics without significant improvement. A random skin biopsy showed lobular panniculitis and he was treated with steroids, but his fever recurred after steroid withdrawal. A second skin biopsy confirmed the diagnosis of SPTL. A bone marrow examination revealed hemophagocytic lymphohistiocytosis. He was successfully treated with cyclosporin A and prednisolone and achieved a complete response after one year of drug discontinuation. Panniculitis-like skin lesions have various etiologies and may present as a clinical mimic of lupus erythematosus panniculitis. The selection of an optimal site for skin biopsy is crucial to avoid erroneous diagnoses and adverse outcomes. We report a case of SPTL in an HIV-positive patient, which illustrates this diagnostic challenge.
皮下脂膜炎样T细胞淋巴瘤(SPTL)是一种罕见的非霍奇金淋巴瘤亚型,表现为脂膜炎样皮肤病变。它常与噬血细胞性淋巴组织细胞增生症同时出现,这是一种危及生命的高炎症综合征。大多数SPTL病例表达αβ T细胞受体(SPTL-AB),口服免疫抑制剂治疗预后良好。我们报告一例37岁男性HIV感染患者,有一年低热病史,双侧大腿有多个压痛性皮下结节,伴有血细胞减少。他接受了几个疗程的抗生素治疗,无明显改善。随机皮肤活检显示小叶性脂膜炎,给予类固醇治疗,但停用类固醇后发热复发。第二次皮肤活检确诊为SPTL。骨髓检查显示噬血细胞性淋巴组织细胞增生症。他接受环孢素A和泼尼松龙成功治疗,停药一年后达到完全缓解。脂膜炎样皮肤病变有多种病因,可能表现为狼疮性脂膜炎的临床模拟。选择最佳的皮肤活检部位对于避免错误诊断和不良后果至关重要。我们报告一例HIV阳性患者的SPTL病例,说明了这一诊断挑战。